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\\nJ

VJjLu > • ®

TWO CASES OF EARLY ATROPHY OF MUSCLES IN

CEREBRAL DISEASE.

R G. FINLBY, M.D.,

Associate Professor of Clinical Medicine in McGill University ; Physician to the

Montreal General Hospital.

Rfiprinted from Ike Montreal Medical Journal, September, 189H.

(.MEDICAL FACUL

,'^'

D-^y^

D^yo ' /•"

///

I'WO CASES

EARLY ATROPHY OF MUSCLES IN CEREBRAL DISEASE.

»y

p. G. FiNi.KY, M.D.,
Associate Professor of Clinical Medicine, McGill University, Physician to the Mont-
real General Hospital.

Muscular atrophy in diseases of the nervous system is ahnost in-
variably due to lesions affecting the cells of the anterior cornua or the
nerves leading from them. (Jlinically such cases are known as
atrophic paralysis, and form a di.stinct and well defined group. In
cerebral disease, or in lesions of the fibres of the cord above the
anterior cornua, atrophy is usually absent, although a moderate degree
of wasting has often been noted in old standing cases of hemiplegia
with contractions and is regarded as due to disease. Of late years,
however, cases have been observed in which muscular atrophy has
been early noted in limbs paralyzed from various forms of cerebral
disease. Contrary to expectation, in some t)f these no changes have
been found either in the anterior cornua or in the peripheral nerves.
With the object of drawing attention to this condition I .submit the
two following cases :

Case I. Tumour of optic thalmus — hemiplegia — atrophy of inuscles
of hand, forearm and leg — anterior cornua and peripheral nerves
normal.

Miss F.,rt>t. .55, was first seen .Tune 21st, 1892. For a month or five weeks past states
that she has had pain over the anteriorpart of the scalp, intermittent, not severe
and present chietly in the morning. She has also had pain in the back of the neck,
most marked on rising from the recumbent posture. Aboiit the same time she
began to experience diiticulty in walking, having a tendency to fall backwards and
her knees giving way under her. She has vomited once or twice half an hour after
her evening meal, without efTort or nausea. There has bci'n giddiness. Her friends
state that she has been growing stout since her illness began, and that her memory
has been failing for three months.

Present Condition. — The patient is well nourished and somewhat stout. Intelli-
gence is fair, but she exhibits no anxiety about her condition, and there is a ten-
dency to laugh easily.

In walking she moves the legs slowly and requires assistance. Tliere is a marked
tendency to fall backward and to the left side.

She is unable to use the hands even for eating. The arms and legs both show
a marked degree of muscular power.

The left labio-nasal fold is not so prominent as the right, and the movements in
the lower part of the face are not (|uite so marked as on the right, but the upper
muscles of the face move normally. Tl\e tongue is protruded straight.

' Read before the Canadian Medical Association, at Montreal, August 28, 1896.

The senHc of position in nornuil iiiid IIutc Ih no iitivxiu of iinii.H and Icks.
SeuMntlon in the ftici- and limb is normal. Hotii icnou Ji>i'l<.s nre uxagfft-rHtcd, iint
tliere Is no anlclc clonns.

The left eye Ih shrunicen and functionlemH, the result of an old injury. The right
optii' disc mIiowh a marked grade of neuritis. The pulse Is IH). Tlie heart, lungs and
urine are normal.

Mental failure and weakness progressed rapidly, an<l on July VM\\ she was
admitted to the Montreal (Jeneral Hospital. She was then (lui e unable to give
any account of herself. She lies on her buck with her head slightly retracted, sleep-
ing a great deal day and night. She answers (|uestions only in monosyllables and in
slow n)e.asured tones after a i)ause of some seconds, relapsing into a soporose condi-
tion when left alone. There is no pain. Urine and fieces are passed in bed.

Tile left arm ind leg are paralysed and llaccid. She is not able to register with
the dynamometer with the left hand, bub registers 'M witli the right. There is some
loss of power in the right limbs.

.July 15. Urine alkaline s.g KKWI : no albumen ; no sugar. Complains of pain in
riglit sterno-niastoid muscle.

July 20. She lies in the same condition, sleeping most of the time, and snoring
loudly. The pain in the neck has gone. There is not much change from day to day.
July 2(1. Wasting of the left thenar eminence was noticed today, and on mea-
suring the following results were obtained :

Right. Left.

Arm !U in. OJ in.

Fore-arm H 74

Hand over thenar eminence 7 flft

There was an inch difTerence in the legs in favour of the right side. No tender-
ness of the muscles or lu-rve trunks present. With the faradic battery a iftuch
stronger current was required to produce contraction of the muscles of the left
hand and forearm, aTid in the respiratory muscles of the face. In the leg there was
slight diminution of electrical irritability to faradism. Tlie galvanic reactions were
not obtained. The atrophy progressed rapidly in the hand, and the thenar and
hypothenar eminences became rtat and depressed. On A\)g. lird, she passed into a
comatose condition, the breathing became rapiil and stei'torous, and death occurred
on the following day (Aug. 4.)

The measurements of the limbs taken after death were as follows :

Kight. Left.

Arm \n. 04

Forearm 8 74

Hand 7 OA

Thigh m HA

Calf 11 wl

During her stay in hospital the temperature was slightly elevated, 08 to 100, and
102i for three days preceding her death. The pulse was also increased in rate, vary-
ing froni 72 to 104.

Tile clinical diagnosis was tumour of the brain, based on the optic
neui'itis, with other cex'obral symptoms. Its locality was regarded as
being in the neighbourhood of the cerebellum, owing to the peculiar
gait. In the absence of any signs of peripheral neuritis, such as
tenderness or loss of sensation, the atrophy of tlie hand was regarded
as due to changes in the anterior cornua of the cord.

Au'r()i'sv.— /yram.— The floor of the third ventricle was full iiiid bulging. The
right optic thalamus was enlarged and infiltrated with a white tumour of the same
colour as the white substance of the cerebrum; its boundaries were ill-defined and
infiltrating. It was rather firmer than the surrounding brain tissue and extended
back as far as the superior vermiform process of the cerebellum. The tumour was

))Iiic(hI ill Milller's fluid fur I'xainiiiiitioii, l)iit duriiiK cIiiiiiki'h i" tlic lalionitory wiix
unfortunately loHt l)efore its exact bouiularieM or inicroHcopic c'laracters were
(letermlned.

The siiliia! cord and a portion of the median nerve at the elbow were also removed
and placed in Miiller's lluid for cx'Mniiuitlon. Sections of the cord at the levels of
the 4th, 5th, (Hh, 7th and Htli cervical and Ist dorsal nerve were 8ubse(|ueiitly made
and stained by VVelKcrt's method and with uarinine. The cells of the anterior
cornua presented no diininutiori in si/c or number ; there was no descending denen-
eratiou and tlie cord was In all respects normal. The median nerve also showed no
sign of dcf;(Mit!ralioii.

The abdominal and thoracic organs were ui.der-weight, but otherwiHe presented
no chauKe of importance.

The cliiuf t'catur(!.s ot" this case wore hemiplejifia and wasting of the

paralysed inu.sch'.s. The wastiiiij; was noted fourteen days after the

limbs hecame paralysed. It afteeted chieHy the thenar muscles of

the hand, where the atrophy was considerable, and to a less extent

the forearm and leg. Ihe limbs were Haccid and there had never

been any irritative sj-mptoms. The wasting in the hand was such as

to suggest a lesion of the anterior cornua. Anatomically, however,

no Tuicroscopic changes were found in the lower motor segment or

even in the p3'ramidal tracts of the cord. During life there was no

evidence of neuritis, sensation having been normal, and no tenderness

of the nerve trunks or muscles was present.

Case II, — Sarcoma of crus cerebri — Hemiplegia and rapid atrophy

of muscles of hand, forearm, arm and shoulder — Autopsy.

I am indebted to Dr. James Sttiwart, of Montreal, for brief clinical

notes of the foUowino; case.

Mr. It., jet. 17. The first symptom noted was loss of col mr vision. He then
sud'ered from severe pain in the head. Weakness in the left ana and leg, gradually
increasing in intensity, set in. The muscles of the thenar and hypothenar
eminences, the forearm, the arm, the deltoid and lower portion of the pectoraiis
major wasted rapidly and death occurred four months from the onset ot symptoms.

Aidopnj/. TUa thenar and hypothemir eminences, the muscles ot the forearm
and arm, the deltoid and lower portion of the pectoraiis major were nmch wasted
on the left side. On removing the brain a greyish, .soft, Hattened growth lying on
and adherent to the right crus cerebri was obsei'vcd. The growth reached from the
anterior border of pons forward to about the level of a line through the middle of
the temporosphenoidal lolic. The growth was ([uadrilateral in shape, 1^' inches
long and li inches broad. The third and fourth nerves lay alongside the tumour,
whilst the optic tract lay beneath the growth. None of the cranial nerves were
involved, a fact which causeil nnich obscurity in localising the growth during life.

Microscopically the tumour proved to be a sarcoma with large vascular spaces.
The upper part of the spinal cord was removed and also a portion of the ulnar nerve.
Sections of the cord at various levels in the cervical region down to and including
the lirst dorsal segment showed the cells of the anterior cornua to be perfectly nor-
mal. There was no degeneration of the lateral columns. The sections were stained
both wiih carmine and by Weigert's method.

Sections of the ulnar nerve were normal. The muscle was not examined.

The chief interest in this ease lies in the fact that a considerable degree of
atrojihy of the muscles of the arm was present, associated with a tumour of the
erus cerebri and without lesions of the anterior cornua or peripheral nerves to
account for it.

Atropliy oF tlu! mu.sclcs is occasiourtlly H»«eii in old crtarH of heini-
plf^nu witli coiitractiirt", ami k'siuiis in tin- anterior cornuu <»r in the
pt'iiphural nervt's have been dfinonstrattnl. Cliaicot first ilnscrilxid
ati'ophy in tlio cells of the anterior cornua at levels corresponding
with the wasted muscles. Dejerine found dej^eneration of the peri-
pheral nerves and reyar<ls this as the soli^ cause of the atrophy.

There is, howevei', a class of eases in which wasting;' occurs early in
the paraly.sed inend)ers and in which no changes either in tin* anterior
cornua or peripheral nerves have l)een present. The wasting cannot
bo attributed to disuse, aa it occurs too early; and again, it may be
present to a consi(leral)le e,\tent in niuseles ordy partially paralysed.

AtKUoin'ictd lj('n'ii>ns. — The pathological conditions in the brain
vary both in site and character. In a considerable proportion of the
cases tuinoiu's have been present, bi.t in others softening or hrenuir-
rhage have e.\iste(i. Bremer and Carson' have collected six (including
tlx'ir own") cases in which a tumour was present. Quincke" has
reported a .sevt>nth aii<l (piotes a case of fiarresi's and one of (iliky's.
Packard, in a paper read at the meeting of the l^i'diatric Society in
Montreal, LSOO, reporttMl a case of a tumour in a child a.s.>-()ciated with
considerable atroph}-, and in both my own cases a cerebral growth
was present.

In Babinski's' case a focus of softening in the centrum ovale minus,
in the course of the psycho-motor Hltres was found. Eisenlohr,
reports two cases, in one of wh'ch a recent, and in the other an old,
heemorrhagic focus in the l)rain was found.

The site of the lesions varies, but all involve some porti' n of the
motor tract. A considerable lunnber of the cases of tumour have
been in the motor cortex, l)ut in otiuirs the paralysis and ensuing
atrophy have resulted from di.sease of the motor tract in the sub-
cortical region and in the internal capsule. The optic thalmus has
al.so been primarily involved with <lamage to the ailjacent internal
capsule.

Secondary degeneration of the pyi-amidal t>'acts and medulla some-
times occurs, an<l also degeneration of the opoosite c/oss pyramidal
tract of the cord and of the direct pyramidal tr.'.ct on the .same side as
the lesion. The (Uigenei'ation of the pyi'amidal tracts is by no means
con.stant and it can therefore have nothing to do with atroph}' of the
muscles.

The most surprising and important fact in th(!se cases of early
muscular atrophy is, however, the absence of changes in the motor
cells of the anterior coi'ima, in the; antei'ior nerve roots and in the
peripheral nerves. This fact is all the more remarkable inasmuch as

Uh' r|(!prcc of atrophy is cit'tt'ii consitluralilc nii<l occurs within n very
.short [M'Hofl of I'.'ur.

The follow iti},' wi iters (l.c) report cascH of rarly nmscular atrophy
witli iiitc^frity of t i." lower inotfir s«'i,Miiciit, tlt'tcriniin'il hy microscopic
I'xaiiiiiiatioii of the (ord ami iicrvcH. (Quincke, JJaKiriski, Kiscnlohr,
(two cases), Hreiner and Carson which, with my own cases, make a
total of seven.

The mu.scles in the few cases in which they have lieen e\amin«'il
present chan<;es similar to those found following atl'ections of the
nerves (v, Babinski, Ki.senlohr l.c).

No very satisfactory explanation of mu.scular atrophy with integrity
(»f the lower motor segment has yet heen oM'ered. (Quincke suggests
the presence of trophic centres in the cortex, hut weie this the case
early atrophy might he expected to occtn- nnich more frticpnuitly.
Babinski (i.e.) and Jotfrey an<l Achard' suggests that the motor cells
of the anterioi' cornua undcirgo dynamic changes, sutficient to intei--
fere with the nutrition of the nnisdes, but not evidenced by anatomi-
cal changes.

SympUmw — The period elapsing between paralysis of the muscles
an 1 atrophy varies considerably. It is often ditlicidt to fix owing to
the fact that wasting is present when the ])atient tiist comes under
observation and has not previously been noticed by him, and again,
its onset may not be ob.served by the physician until it has reached a
considerable degree. The most rapid onset is recorded by Borgherini'
in which muscular atrophy (anunniting to a difference of 1 cm. in the
arm, 5 cm. in the forearm, 5 cm. in the thigh and 1 cm. in the leg)
was noted on the third day after an attack of hemiplegia, but it is
usually observed about three^ or four" weeks aft(;r the onset of paralysis.
Although paralysis is usually complete, atrophy may occur where
paresis' only is present.

The muscles of the aiTu usually present an earlier and greater
degree of wasting than the leg. The nui.scles of the arm, forearm,
shoulder and hand, may all be affected, and in one of Quincke's cases
wasting began in the shoulder and arms, the thigh and calf. When
the hand is affected, the atrophy may reach a considerable degree,
the eminences of the thenar and hypothenar groups being flat or
depressed, and the first interosseous muscle also showing a consider-
able degree of atrophy.

The diffei'ence in the size of the limbs varies from 4 cm. to .5 cm.
In the hands, although the difference in measurement may be slight,
the atrophy seems to reach at times a higher grade than in othei"
muscles. From these facts it appears that the muscles most affected

arc tlioMit which .suflbr iiutst in ct'i-ttliml |iiii-nly.si.H, attil in v\ !iicli thr
nu)Vt!nu'nt.s perfoniUMl aiv complex iiiid highly (liHlTciitiuti'il.

Th«! kruit'-jcrks in the ca.v«s undttr disoussion are UHiuilly iiicreasofl
mill t'not clonus has hccn noti'd, facts which support tht; view of
intcj^rity ol the hnvcr motor scjjfincnt.

h'/crlriral. lit'iirthms. — In thf tVw cases t^xaniined the electiical
reactions have shown diminished faradic contractility correspondiiif^
to the wastinj^ (Qnincke), In my first case, liowever, the faradic
irritaltility was mnch lowert-d and stronjf currents were required to
produce contraction. The jfalvanic irritaliility has shown slif^ht
(|Ualitativ(! and (piantitative chanjjes, hut never the slow muscular
movements seen in nervo degeneration.

Summarising we may state that in a certain small proportion of

cases of cerehral disease muscular atrophy occurs early and if present

in the hand may reach a considerahle degree, and that in these cases

no anatomical changes are ohservahh' in the lower motor segment.

' Hremer nnd CarHon, Am. Jour. Mid. Sc, 1851, 1, liJJi ♦

niuinckc, D.-iit. Arcli. Kliii. Mpi\., 42, 41)2.

•' Hiil)iiiski, C. r. (If la Soiictt) tU- Uiologie, 188(1, 76.

■• Eisciilolir, alwtract in Vircliow's Jahrt'sbLTicht d. Gch. Med., ISUl, 2, i:iO.

» Arch, do Mi-d., Ecp., 181)1.

« Deut. Arch. Kliii. Med., 15.

7 Elsciilolir I. c.

" EisuiiUthr, Quincke I.e. [three cases.)

" Quincke I.e., Case I. Bremer and Carson I.e.